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Rheumatology and Immunology Therapy PDF

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Blackplate(1,1) A A beta binations is strongly influenced by ethni- city. In addition to expression on erythro- cytes, AB blood group antigens are ex- Beta-amyloid protein pressed on a variety of other cells, includ- 3 ing lymphocytes and platelets. Patients with erythrocytes which express type A antigen on their surface have serum anti- A beta 2m bodies against type B red cells, and vice- versa. Patients with type O blood have Amyloidosis, beta -microglobulin antibodies against both A and B antigens, 3 2 andpatientswithtypeABbloodhaveanti- bodies against neither. If, in accidental transfusion, type A blood AA isinjectedintopersonswithtypeBblood, theinfusedredcellswillberapidlyhemo- Arachidonic acid lyzed by antibodies in the recipients’ 3 serum and will cause a severe transfusion reaction which can be life threatening. Likewise, type B red cells are destroyed AA metabolites by anti-B antibodies in type A blood. TypeObloodcanbetransfusedinpersons with type A, B, or O blood (and thus pa- Arachidonic acid metabolites 3 tients with type O blood are considered "universal donors") unless there is incom- patibilitywithrespecttosomeotherblood ABO blood group system groupsystem.PersonswithtypeABblood canreceivetypeA,B,orOblood(andare Definition thus "universal recipients"). The ABO antigens are developed before birth and TheABObloodgroupsystemisamethod remain throughout life. of classifying human blood based on cell surface antigens. References Full Text 1. Reid MR, Lomas-Francis C (1997) The TheABObloodgroupsaredefinedbythe blood group antigen facts book. Academic presence of the type of surface sugar, Press, NewYork whichdeterminesimmunogenicity.There- lative distribution of the blood type com- Blackplate(2,1) 2 ABObloodgroupsystem,transfusionreactions ABO blood group system, fusion. Anaphylactic transfusion reactions transfusion reactions arecharacterizedbytherapidonsetofana- phylaxis(shock,hypotension,angioedema and respiratory distress) following a trans- Synonyms fusion containing plasma. These reactions Immunologic blood tranfusion reactions. usually occur in people who are IgA defi- cient who exhibit the presence of class- Definition specific IgG, anti-IgA antibodies. Urticar- ial transfusion reactions are due to the in- There are several types of transfusion re- teraction of allergenic substances in the actions which include febrile nonhemoly- plasma of donor blood products with pre- tic, acute hemolytic, delayed hemolytic, formed recipient IgE antibodies. This will anaphylactic and urticarial. These occur resultinthereleaseofhistaminefrommast in 1–6% of all blood transfusions and are cells or basophils, causing urticaria. morecommoninpatientswithpre-existing oncologic or hematologic disorders. Fe- brile nonhemolytic transfusion reactions Differential Diagnosis are the most common type of transfusion Symptoms characteristic of the various reaction and are believed to be caused by transfusion reactions including anaphylax- cytokines (i.e. IL-1, IL-8 and TNF-α). is,angioedema,shock,urticaria,hemolytic These cytokines are present in stored anemiaandDICmaybefromothercauses blood products and increase over time. and should be thouroughly investigated. Also possible is an interaction between Additional problems to consider include donor leukocytes and recipient antibodies food allergies, cardiogenic pulmonary which might cause the release of IL-1, re- edema,sepsisandcoldagglutininimmune sulting in a fever during the transfusion. hemolysis. These transfusion reactions can also occur after platelet transfusions and thus, another proposed etiology involves the re- References lease of platelet-derived CD154 (CD40 li- 1. Sazama K, DeChristopher PJ, Dodd R gand), which is capable of producing (2000) Practice parameter for the recogni- proinflammatory cytokines from fibro- tion, management, and prevention of ad- blasts, epithelial cells, and endothelial verse consequences of blood transfusion. cells. Acute hemolytic transfusion reac- College of American Pathologists. Arch Pathol LabMed124(1):61–70 tions occur when the donor red blood 2. Silvergleid AJ (2003) Immunologic Blood cellsaredestroyedbythepreformedrecip- TransfusionReactions.http://www.uptodate. ientantibodies.Thesereactionsareusually com due to ABO incompatibility (group O re- cipient is given non-group O red blood cells), usually due to clerical error, but may also result from acquired alloantibo- Abortion, anticardiolipin dies (i.e. anti-Rh, anti-Jka). antibody Delayed hemolytic transfusion reactions result from re-exposure to foreign red blood cell antigens causing an antibody Synonyms response. These reactions may occur fol- lowing exposure during previous organ Pregnancy loss, fetal loss, antiphospholi- transplantation, pregnancy or blood trans- pid syndrome, APS. Blackplate(3,1) Acanthosisnigricans 3 Definition A shown to providebenefit inpreventingre- Pregnancy loss associated with anticardio- current miscarriages. lipin antibodies. References Full Text 1. Branch DW, Porter TF, Paidas MJ, Belfort Pregnancy loss is associated with the pre- MA, Gonik B (2001) Obstetric uses of senceofmaternalantiphospholipidantibo- intravenous immunoglobulin: successes, failures, and promises. J Allergy Clin dies (anticardiolipin antibodies and lupus Immunol 108:S133–8 anticoagulants). The type of pregnancy 2. Scott JR (2003) Immunotherapy for recur- loss most frequently associated with anti- rent miscarriage. Cochrane Database Syst phospholipidantibodiesistheunexplained RevCD000112 deathofamorphologicallynormalfetusat or beyond 10 weeks gestation. Spontane- ousabortionsbefore10weeksmayalsobe Acanthosis nigricans associated with antiphospholipid antibo- dies. Fetal death associated with antipho- spholipid antibodies is most likely due to Synonyms insufficient uteroplacental blood flow and Keratosis nigricans. fetal hypoxia. Definition References Accompanies obesity, diabetes mellitus, 1. Branch DW, Khamashta MA (2003) Anti- excess corticosteroids, genetic mutations, phospholipidsyndrome:obstetricdiagnosis, evironmental factors nicotinic acid, estro- management,andcontroversies.ObstetGy- necol 101:1333–44 gens, corticosteroid, tumors: adenocarci- noma, pineal tumors, amongst other pa- thology. A brown thickening of skin which may progress to a leathery, warty Abortion, intravenous or papillomatous appearance do to papil- immunoglobulin lary hypertrophy, hyperkeratosis, and in- creased epidermal melanocytes. Most Synonyms common sites are the axillae and flexural Pregnancy loss, fetal loss, IVIG. Definition Use of intravenous immunoglobulin (IVIG) to prevent pregnancy loss due to immunological abnormalities. Full Text Intravenous immunoglobulin has been used in an attempt to prevent pregnancy loss in women with the antiphospholipid syndrome and unexplained recurrent mis- carriages. In the antiphospholipid syn- drome, IVIG was no more effective than Acanthosis nigricans. heparin and low-dose aspirin. In rando- Figure1 Acanthosis nigricansona16 mized controlled trials IVIG has not been year-oldgirl(courtesyofSateHamzaMD). Blackplate(4,1) 4 Acarbose surfaces of the neck, groin, belt line, dor- type II diabetes mellitus. A study of 1429 sumofthefingers,mouth,periareolaeand subjects with impaired glucose tolerance umbilicus. randomized to either acarbose or placebo foundthatacarbosesignificantlydecreased Differential Diagnosis the risk of progression to diabetes and re- Benign/pseudoacanthosis nigricans – versedglucoseintolerancemorefrequently mostlyontheobesespeculatedtocorrelate thanplacebo.Acarbosemayhavearolein with increased fasting plasma insulin le- the prevention of diabetes in some at-risk vels. Sometimes benign disease relates to patients. rare autosomal dominant trait without un- Dosage derlying endocrinopathy. May sometimes be drug induced. Rangesfrom50mgto100mgtakenthree times daily, taken with the start of each References meal. 1. Habif T (1996) Clinical Dermatology, 3rd Contraindications ed.MD Consult Acarbose may cause gastrointestinal dis- tress including flatulence, cramping and diarrhea due to the presence of undigested Acarbose carbohydratesinthebowel.Therefore,pa- tients with colonic obstruction or inflam- Trade Names matory boweldisease,orpatients prone to Precose (US), Glucobay, Gluconase, Glu- developing intestinal obstructions, should mida, Precose, Prandase. not take this drug. Elevated hepatic trasa- minases have also been observed with Class and Category acarbose use so patients with cirrhosis Alphaglucosidaseinhibitor,anti-hypergly- should avoid the drug. Patients with dia- cemic agent. betic ketoacidosis should not be adminis- tered acarbose. Indications Adverse Reactions The primary use of acarbose is the man- agementofhyperglycemiainpatientswith The most common adverse reactions are GI symptoms related to undigested carbo- hydrates traversing the gut. Higher doses ofacarbosehavebeenassociatedwithele- vated hepatic transaminases and occasion- allyhyperbilirubinemiaandjaundice.Rare casesoffulminanthepaticfailurehavenot been shown to be clearly related to acar- bose use. Drug Interactions Drugsthatimpairthemechanismofaction ofacarboseorinterferewiththeinteraction of acarbose with the gut epithelium could reduce the effectiveness of the drug. Acanthosis nigricans. Therefore,intestinalanti-absorptiveagents Figure2 Acanthosis nigricansaxilla. Blackplate(5,1) Acetaminophen 5 (i. e. charcoal) and replacement pancreatic Accessory cells A enzymes (pancreatin, amylase) should not be taken. Withdrawal of hyperglycemic Definition agents such as cortcosteroids in patients Nonlymphoid cells which are required to takingacarbosecouldprecipitatehypogly- induce a T-cell dependent immune re- cemia, so blood glucose should be moni- sponse. tored closely in these situations. Full Text Mechanism of Action Accessory cells are nonlymphoid cells such as macrophages, dendritic cells, and Acarbose is a complex oligosaccharide Langerhanscells(epithelialdendriticcells) thatcompetitivelyinhibitsgutenzymesre- that function to present antigens to MHC- sponsible for metabolizing complex restrictedTcells.Theyarenotantigenspe- starches and oligosaccharides into absorb- cific and they present different costimula- ableglucose.Asaresult,theabsorptionof torymolecules,eithersolublecytokinesor ingested carbohydrates is delayed and memebrane bound peptides, with the anti- post-prandial hyperglycemia is reduced. gen to the T-cells. The specific combina- Acarboseinhibitsthefunctionofpancreat- ic α-amylase in the intestinal lumen and tionofantigen,MHC-restrictedTcell,and intestinal membrane-bound α-glucosidase expressed costimulatory molecules deter- mines the specific immune response. hydrolase enzymes. References Definition 1. Yung RL (2000) Geriatric rheumatology, changes in immune function with age. Acarbose is a unique anti-hyperglycemic RheumDisClin North Am26:455–73 agent which reduces post-prandial hyper- glycemia by inhibiting intestinal enzymat- icactivityresponsibleformetabolizingin- ACE gested carbohydrates into absorbable sugar. Since it has a unique mechanism of action, acarbose can be used in combi- Angiotensin Converting Enzyme nation therapy with other medications to 3 treattypeIIdiabetes,includingsulfonylur- eas and metformin. Acetaminophen References Synonyms 1. (2002)Acarbose.In:NissenD(ed)Mosby’s Paracetamol. Drug Consult13thed.Mosby,St.Louis 2. Chiasson JL, Josse RG, Gomis R, et al Trade Names (2002) Acarbose for prevention of type 2 diabetes mellitus: the STOP-NIDDM ran- Tylenol. domised trial. Lancet359:2072–7 3. Mertes G (2001) Safety and efficacy of Class and Category acarboseinthetreatmentofType2diabetes: Analgesic; antipyretic. data from a 5-year surveillance study. Dia- betes ResClin Pract52:193–204 Indications Arthralgia; dental pain; dysmenorrhea; Blackplate(6,1) 6 Acetylcholinereceptorantibodies fever; headache; mild pain; myalgia; os- alone or in combination with other medi- teoarthritis. cations for the treatment of pain, fever, sinus symptoms, and the symptoms of Dosage upper respiratory infections. Acetamino- Adults and children age 12: 325–650 mg phen is available over the counter and by orally or per rectum every 4–6 hours as prescription. needed; or 1000 mg orally or per rectum 2–4 times per day. Maximum of 1000 mg References per dose and 4000 mg per day. Children 1. RobertsLJ2nd,MorrowJD(2001)Analge- andinfants:10–15mg/kgorallyorperrec- sic-antipyretic and antiinflammatory agents tumevery4–6hours.Maximumof5doses and drugs employed in the treatment of in24hours.Neonates:10–15mg/kgevery gout. In: Hardman JG, Limbird LE (eds) 6–8 hours. Pharmacological Basis of Therapeutics, 9th ed.McGraw-Hill, NewYork,pp687–731 Contraindications Relative contraindications: severe liver Acetylcholine receptor disease, alcoholism, and prior hypersensi- tivity reactions to acetaminophen. antibodies Adverse Reactions Autoantibodies, myasthenia gravis Hepatoxicity may occur after acute over- 3 dose, or with chronic excessive intake. Acetaminophen in combination with other analgesics may contribute to analge- Acetylsalicylic acid sicnephropathy.Methemoglobinemiawith acute overdose. Aspirin 3 Drug Interactions Diflunisal increases acetaminophen drug levels. Regular ethanol consumption in- Achilles bursitis creasesriskofacetaminophenhepatictox- icity. Synonyms Mechanism of Action Retrocalcaneal bursitis, adventitial bursi- Mechanism of action not completely tis, superficial calcaneal bursitis, pump known. Inhibits cyclooxygenase in the bump. central nervous system which may contri- bute to analgesic and antipyretic proper- Definition ties. Inflammationofeitherofthe2heelbursa. One bursae (retrocalcaneal) lies between Definition the Achilles tendon and the calcaneus, Acetaminophen isapara-aminophenolde- and the second bursae (superficial calca- rivative with analgesic and antipyretic ac- neal) overlies the Achilles tendon. Often tions; but no significant anti-inflammatory mistaken for Achilles tendonitis, although activity.Acetaminophenisthedrugoffirst patients may have both (Haglund’s syn- choice for treating pain and fever in chil- drome). Overuse injury and low cut dren. In adults, acetaminophen is used shoes are predisposing risk factors. Blackplate(7,1) Achillestendonitis 7 Pharmacological Achilles tendonitis A Non steroidal anti-inflammatory drugs mayoftenbeusefultotreatpainassociated Synonyms with bursal inflammation and pain. Some physicians perform glucocorticoid injec- Achilles tendinitis. tionsintothebursa.However,directinject into the Achilles tendon is generally not Definition recommended as this procedure has been Inflammation of the Achilles tendon. Eti- associated with tendon rupture. ology is usually mechanical, including in- correct running technique, overuse, or Non-pharmacological poorly fitting shoes. Other causes include rheumatic conditions predisposing to ten- Application of ice and/or heat is simple donitis such as spondyloarthropathies. and may be effective in reducing inflam- mation. Ultrasound also can be used to treat this condition. Shoe inserts placed at Therapy the posterior heel or switching to shoes Pharmacological without heels (e.g. open backed sandals) Non steroidal anti-inflammatory drugs can also be helpful. mayoftenbeusefultotreatpainassociated with tendon inflammation and pain. Some General Therapeutic Measures physicians perform glucocorticoid injec- tions at the site of tendon inflammation, Lifestyles which may also be therapeutic. Caution Rest and gentle stretching are often help- must be taken not to directly inject into ful.Itisalsoimportanttoavoidingexacer- the tendon itself (often associated with a bating activities, such as walking or run- high degree of resistance), as this may in- ning uphill. crease the risk of tendon rupture. Prognosis Non-pharmacological Generallygood,andoftenthisconditionis Many physicians recommend application self-limited. However, recurrence is com- of ice in the first 24–48 hours following mon. Surgical resection of the bursa (and atendoninjury.Heatisoften usedfollow- any associated bony prominence of the ingthattime,althoughnooptimalregimen calcaneus) is rarely performed for refrac- forthesemodalitiesexist.Althoughrestor tory cases. evenimmobilization(oftenwithasplintor sling) of an anatomic structure may speed recoveryifthetendoninflammationisdue References to an overuse injury, patients should con- 1. Mazzone MF, McCue T (2002) Common tinue passive range of motion. conditions of the achilles tendon. Am Fam Physician 65(9):1805–10 General Therapeutic Measures Lifestyles Achilles tendinitis Rest and gentle stretching are often help- ful.Itisalsoimportanttoavoidingexacer- bating activities, such as walking or run- Achilles tendonitis ning uphill. 3 Blackplate(8,1) 8 Achondroplasia Prognosis 2. Ruddy S, et al (eds) (2001) Kelley’s Text Generallygood,andoftenthisconditionis book of Rheumatology, 6th ed. WB Saun- ders,Philadelphia self-limited. However, recurrence is com- 3. Cecil RL, Bennett JC, Goldman L (eds) mon. (2000)CecilTextbookofMedicine,21sted. WBSaunders,Philadelphia References 4. Larson PR (2003) Williams Textbook of 1. Mazzone MF, McCue T (2002) Common Endocrinology, 10th ed. WB Saunders, conditions of the achilles tendon. Am Fam Philadelphia Physician 65(9):1805–10 Acid ceramidase deficiency Achondroplasia Definition 3Faber’s disease Most common chondrodysplasias in hu- mans. Autosomal dominant trait (fibro- blast growth factor receptor-3 gene local- Acid maltase deficiency ized to 4p16.3) with sporatic new muta- tions observed in 80% increasing in fre- Definition quency with paternal age. Estimated fre- quency of 1/14,000–40,000 live births. Enzyme deficiency of alpha glucosidase Characterized by short-limbed (rhizome- found in lysosomes that catalyze glucose lia), short trident hands, redundant skin, release from complex sugars. Located on normal-sized trunk, waddling gait, thora- chromosome 7q21-23, alpha-glucosidase columbargibbous(severekyphosis),meg- deficiency is thought to be transmitted as alocephaly with frontal bossing, low nasal an autosomal recessive trait that affects bridge. Radiographs: small cuboid-shaped three distinct age groups. Infant form, vertebral bodies, progressive narrowing of Pompe’s disease, occurs between 0–2 the lumbar interpedicular distance, small yearsofagecausingweakness,hypotonia, iliac wings, narrow sciatic notches. andCHFinvariablyleadingtodeath.Mas- sive gloycogen deposits can be found in Prognosis cardiac, hepatic, neural and muscular tis- Homozygousinfantstypicallydieininfan- sue. Early childhood forms present with cyfromrespiratoryinsufficiencysecondto muscle weakness, proximal > distal, with asmallthoraxwhilemeanadultmalesand progressive respiratory failure leading to females are 130 and 120 cm respectively. deathinthe30s.MaysimulateDuchenne’s Despite problems achondroplasia does not muscular dystrophy with firm, rubbery incur a shorter life expectancy nor poorer gastrocnemius and deltoid muscles, gas- health. Complications arise from hydro- trocnemius hypertrophy and the Gowers’ cephalus, neuronal compression of such sign. Late toe walking develops after systems as brain stem, spinal cord, or anklecontracturesandlumbarlordosiscre- nerve roots via minimal impingement by ates an unsteady gait. a disk or osteophyte is common. Adult onset, 30–40s, with muscle weak- ness noted to be a progressive myopathy, References onethirddeveloprespiratorycompromise. 1. Stedman’s Medical Dictionary. http://www. Proximalpelvicgirdleorthoraxicintercos- stedmans.com tals and diaphragm muslces are mostly in- Blackplate(9,1) Acne 9 volved in a groupwise fasion although in- 4. Cotran RS, et al (eds) (1999) Robbins A dividual muscle parts may be affected. Pathologic Basis of Disease, 6th ed. WB Saunders,Philadelphia Often adults are mislabeled as polymyosi- 5. Koopman WJ (ed) (2001) Arthritis and tis or late-onset muscular dystrophy. Allied Conditions. Williams & Wilkins, Creatine phosphokinase is usually ele- Baltimore vated. Venous lactate concentrations in- creasenormallyafterforearmischemicex- ercise. EMG changes consistent with in- ACL tense electric irritability, myotonic dis- charge without a clinical picture of myo- tonia. Deficient or decreased alpha-gluco- Anticardiolipin antibodies 3 sidase activity in muscle or leukocytes all butdefinesthedisease.Biopsyshowsvac- uolar myopathy with elevated glycogen Acne and acid phosphatase staining. Small foci of acid phosphatase can be found in mus- cle fibers without vacuoles. Electron mi- Synonyms croscopyshowsglycogendepositioninin- Acne vulgaris. volved muscle. Definitive diagnosis via enzyme activity levels is needed. Definition Acneisaskindiseaseofthepilosebaceous Therapy follicles.Hairfolliclesdevelopretentionof Non-pharmacological keratin and sebum, which leads to inflam- Nocturnal respiratory support, with resis- mation and follicular plugging. This leads tiverespiratorymuscleexercises,areoften toformationofpapules,comedones,cysts, helpful. pustules, and nodules. Acne most com- Severe early respiratory muscle failure re- monlyaffectsthefaceandtrunkofadoles- sults in early bilevel positive airway pres- cents.Mostpatientsoutgrowacneafterad- sure or continuous positive airway pres- olescence,howeversomepatientscontinue sure dependency with possible long term to have symptoms in their twenties and home ventilation required. thirties.Thereisastronggeneticpredispo- sition in most patients. Stimulation of se- General Therapeutic Measures baceous glands by excessive androgens Diet and metabolism of sebum by Proprioni- High protein. bacterium acnes, forming free fatty acids, may lead to development of acne. Prognosis Therapy See above. Pharmacological References Topical therapy with benzoyl peroxide, 1. Ruddy S, et al (eds) (2001) Kelley’s Text topical retioids, and topical antibacterials book of Rheumatology 6th ed. WB Saun- may reduce levels of Proprionibacterium ders,Philadelphia acnes, and prevent new lesions from de- 2. Goetz CG (1999) Textbook of Clinical veloping. Neurology.WB Saunders,Philadelphia Oral antibiotics, including tetracycline, 3. Noble J (ed) (2000) Textbook of Primary CareMedicine,3rded.ElsevierScience,St. doxycycline, minocycline, clindamycin, Louis and erthythromycin reduce the number of Blackplate(10,1) 10 Acnearthritis References bacteriaontheskin,thuspreventingdevel- opment of new lesions. Oral contracep- 1. Odom RB, James WD, Berger TG (eds) tives and spironolactone act to inhibit (2000) Acne: Andrews’ Diseases of the Skin. Clinical Dermatology, 9th ed. WB ovarian androgen secretion, and thus de- Saunders,Philadelphia, pp284–93 crease sebum production. However, oral contraceptives which have androgenic properties may worsen acne. Isotretinoin Acne arthritis is approved for treatment of severe cyctic acne. This agent is highly teratogenic and may cause hypertriglyceridemia, thus ex- Arthritis, acne treme precautions must be undertaken to 3 monitor for pregnany and elevated trigly- cerides. Acne conglobata Non-pharmacological Synonyms Abrasive cleansers, astringents, and soaps Nodulocystic acne. are not typically helpful, and may in fact lead to irritation and drying of the skin. Definition Local surgical treatment may be used in Acne conglobata is a severe form of acne which a follicle is incised with a scalpel, in which the lesions are inflammatory co- and the contents of the leson are ex- medones,papules,orpustules.Thelesions pressed. Corticosteroids may be injected are located on the face, forehead, and in cysts, inflammatory papules and pus- neck, and may communicate and form tules; however atropy and hypopigmenta- cysts and sinus tracts. The disease is tion of the skin may result if the steroid is most common in teenaged males. Acne not diluted with sterile saline. conglobata is often chronic, and may lead to severe scarring. The follicular triad syndrome includes acne conglobata, General Therapeutic Measures hidradrenitis suppurativa, and dissecting cellulitis of the scalp. Musculoskeletal Diet symptoms similar to the features of sero- Despite myths about foods exacerbating negativespondyloarthropathiesmaybeas- acne (e.g. greasy foods and chocolates), sociated with this triad, including periph- there is no data to support this. eral arthritis and sacroilitis, often present- ingconcurrentlywithaflareofskininvol- vement. Lifestyles Patients are discouraged from manipulat- Therapy ing lesions, as this may worsen scarring. Pharmacological Topical therapy using benzoyl peroxide Prognosis may be utilized, however more aggressive therapy is usually considered early to The majority of patients will have resolu- avoidscarring.Tetracycline,erythromycin, tion of symptoms after adolescence. Early or minocycline may be used as systemic treatmentmay help prevent emotional dis- therapy.Isotretinoin1–2mg/kd/dayispre- tress and scarring. scribed for five months’ duration. If the

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